Electrophysiology in some animal and human hereditary diseases involving the retinal pigment epithelium.
The present paper surveys slow electrophysiological responses recorded by a d.c. technique in some hereditary eye diseases involving the retinal pigment epithelium (RPE) in animals (English setter dogs and Polish Owczarec Nizinny (PON) dogs with ceroid lipofuscinosis and Briard dogs with a slowly progressive rod-cone dystrophy associated with RPE inclusions) and in humans (Best's disease). The electroretinogram c-wave was typically either decreased in amplitude, lacking or replaced by a negative wave. These c-wave changes could be seen at fairly early stages of disease, when the a- and b-waves of the electroretinogram were still within normal limits.
Nilsson
S E
SE
Department of Ophthalmology, University of Linköping, Sweden.
Wrigstad
A
A
D000293:Adolescent; D000328:Adult; D000818:Animals; D002648:Child; D004283:Dog Diseases / Q000235:genetics / Q000473:pathology / Q000503:physiopathology*; D004285:Dogs; D004594:Electrophysiology; D004596:Electroretinography; D006801:Humans; D008268:Macular Degeneration / Q000235:genetics / Q000503:physiopathology; D008875:Middle Aged; D009472:Neuronal Ceroid-Lipofuscinoses / Q000235:genetics / Q000503:physiopathology / Q000662:veterinary; D010857:Pigment Epithelium of Eye / Q000503:physiopathology* / Q000648:ultrastructure; D012164:Retinal Diseases / Q000235:genetics / Q000503:physiopathology* / Q000662:veterinary*; D012174:Retinitis Pigmentosa / Q000235:genetics / Q000503:physiopathology / Q000662:veterinary